d) air is eventually forced through the TEF and creates abdominal distension

3. Diagnosis

1) some are diagnosed on prenatal ultrasound

2) most are diagnosed after birth

3) if suspected then a rigid nasogastric tube should be placed and a CXR should be obtained which will demonstrate blind esophageal pouch

4) can also use water soluble contrast to study pouch to delineate the anatomy

5) if a right aortic arch is suspected, then echocardiography should be performed

4. Treatment- Preoperative Management

1) place baby in the semisitting position

2) place sump tube for control of saliva or use frequent suctioning

3) resuscitate in the ICU, correct electrolyte and acid base status

4) antibiotics are given

5) mechanical ventilation if required

a) in patients with increased pulmonary resistance, high frequency ventilation with low mean airway pressure may be more effective than conventional ventilation

b) lower mean airway pressures should help control abdominal distension

5. Surgical Treatment

1) surgical approach and timing of surgery depend upon the size of the infant and condition

2) primary repair is preferable for almost all infants unless weight is less than 1500 g or they have associated severe respiratory distress syndrome for which delayed primary or staged repair should be completed 1-8 weeks after birth

3) some infants may be delayed secondary to critical congenital cardiac disease

4) most patients do not require gastrostomy before thoracotomy unless a staged procedure is planned

5) in patients that require high ventilatory airway pressures, a gastrostomy may become a source of an air leak exacerbating respiratory status

6) if the infant has an associated doudenal atresia or imperforate anus, these defects are corrected first then the esophageal fistula (author had no deaths in 24 patients with this sequence)